Abstract:
BACKGROUND:Neuroendocrine cell hyperplasia of infancy (NEHI) is a recently described children's interstitial lung disease (chILD) disorder of unknown etiology. It manifests clinically with tachypnea, retractions, hypoxemia, and crackles. The characteristic radiographic appearance consists of pulmonary hyperexpansion and ground-glass densities on high-resolution computed tomography (HRCT). Lung histology shows hyperplasia of bombesin-immunopositive neuroendocrine cells within distal bronchioles and alveolar ducts without other identifiable lung pathology or developmental anomaly. METHODS:We describe four families with multiple siblings diagnosed with NEHI. Cases were identified at three pediatric centers. Inclusion criteria included clinical findings consistent with NEHI, lung biopsy confirmation in the index case, and a diagnostic HRCT or biopsy in other siblings. RESULTS:Each family had a proband diagnosed with NEHI based upon pathologic review, and at least one additional sibling diagnosed either by pathologic review or HRCT. All patients presented between 2 and 15 months of age. Both male and female children were affected. The majority of the patients underwent both HRCT and lung biopsy. There were no deaths among affected children. No environmental exposures or other potential etiologies were identified as a cause of presenting symptoms. CONCLUSIONS:The familial occurrence of NEHI suggests the possibility of a genetic etiology for this disorder and highlights the importance of taking a complete family medical history for infants presenting with a suggestive clinical picture. Identification of familial NEHI patients allows for the opportunity to further our understanding of this disorder, its natural history, the phenotypic spectrum, and potential genetic causes.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Popler J,Gower WA,Mogayzel PJ Jr,Nogee LM,Langston C,Wilson AC,Hay TC,Deterding RRdoi
10.1002/ppul.21219subject
Has Abstractpub_date
2010-08-01 00:00:00pages
749-55issue
8eissn
8755-6863issn
1099-0496journal_volume
45pub_type
杂志文章abstract::Since persistent pulmonary hypertension of the newborn (PPHN) often occurs as a life-threatening illness, it would be advantageous to identify the highest-risk infants within the first 24 hours of life so that transfer to centers with extracorporeal membrane oxygenation (ECMO) or high-frequency ventilation can be faci...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950050103
更新日期:1988-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
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更新日期:2006-04-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
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更新日期:2013-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.1950200506
更新日期:1995-11-01 00:00:00
abstract:BACKGROUND:Community-acquired pneumonia (CAP) is a leading cause of childhood death. There are few published reports of radiographic findings among children with severe CAP. OBJECTIVE:To describe chest X-ray (CXR) findings and assess association between these radiographic findings and pneumococcal isolation in childre...
journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ppul.21287
更新日期:2010-10-01 00:00:00
abstract::The collection of sputum for microbiological examination in young cystic fibrosis patients can be very difficult. However, a knowledge of bacterial flora colonizing the patient's airways is of paramount importance for proper antimicrobial therapy. It is also known that cystic fibrosis patients colonized by Pseudomonas...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10352
更新日期:2003-10-01 00:00:00
abstract::Dysfunctional swallowing is an uncommon, but important cause of bronchiectasis. We describe a child with a brainstem tumor, who developed bronchiectasis caused by chronic aspiration secondary to a dysfunctional swallow. The case highlights the importance of thorough and repeated evaluation before a diagnosis of idiopa...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21152
更新日期:2010-02-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24123
更新日期:2018-12-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20305
更新日期:2005-12-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950050407
更新日期:1988-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950110315
更新日期:1991-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2008-12-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199805)25:5<299::aid-ppul2
更新日期:1998-05-01 00:00:00
abstract::Obstructive sleep apnea syndrome (OSAS) has been associated with reduced neurocognitive performance in children, but the underlying etiology is unclear. The aim of this study was to evaluate the relationship between hypoxemia, respiratory arousals, and neurocognitive performance in snoring children referred for adenot...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10453
更新日期:2004-04-01 00:00:00
abstract::To determine whether doses of ipratropium bromide (IB) greater than those usually administered by aerosol (75-250 micrograms) give a greater degree of protection from exercise-induced asthma (EIA) in children, 12 patients with chronic asthma, ages 7-13 yr, were challenged with methacholine and exercise after inhalatio...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950060205
更新日期:1989-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23538
更新日期:2017-03-01 00:00:00
abstract::Campomelic dysplasia is a generalized disorder of cartilaginous growth and development, leading to early death from pulmonary insufficiency. We describe the airway dynamics as observed bronchoscopically in two affected infants. Both infants demonstrated anatomic compromise of the upper airway and diffuse laryngotrache...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030514
更新日期:1987-09-01 00:00:00
abstract::Airway obstruction in children is a rare, but difficult clinical problem, with no clear agreement on optimal therapeutic approach. Stenting of the airway has been used successfully in adults, and is an attractive alternative in children. Fundamental differences of pediatric compared to adult use include the benign nat...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.20790
更新日期:2008-04-01 00:00:00
abstract:INTRODUCTION:The prevalence of both childhood obesity and childhood asthma has increased dramatically over the past few decades. Little is known concerning the role of body composition and lifestyle influences on airway health in children. PURPOSE:To determine whether body composition, fruit and vegetable intake (FV) ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21393
更新日期:2011-05-01 00:00:00
abstract::One hundred fifty-four children with recurrent or chronic infections of the lower respiratory tract compatible with the diagnosis of primary ciliary dyskinesia (PCD) were evaluated for the presence of ultrastructural ciliary abnormalities. Studies were performed on multiple samples of respiratory mucosa obtained by na...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950100104
更新日期:1991-01-01 00:00:00
abstract::Twenty patients with cystic fibrosis (CF) were trained to mobilize intrabronchial secretions by a new method of positive expiratory pressure mask (PEP mask) physiotherapy (PT). Patients repeatedly expired forcefully through the PEP mask; expiratory resistance was varied by eight different internal diameter resistors a...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950020608
更新日期:1986-11-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23757
更新日期:2017-10-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22593
更新日期:2013-03-01 00:00:00
abstract::Although airway obstruction and chronic endobronchial infection have long been recognized as major factors in the pathogenesis of lung disease in cystic fibrosis (CF), only recently has it been recognized that the inflammatory process itself may be responsible in a major way for destroying the lungs. The most characte...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199708)24:2<137::aid-ppul1
更新日期:1997-08-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20505
更新日期:2006-12-01 00:00:00
abstract:BACKGROUND:There is minimal literature available on the long-term outcome of pediatric non-cystic fibrosis (CF) bronchiectasis. AIM:To document 5-year outcomes of children with chest computerized tomography (CT) scan diagnosed bronchiectasis from a tertiary New Zealand (NZ) respiratory clinic. METHODS:Review of a cli...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21331
更新日期:2011-02-01 00:00:00
abstract:BACKGROUND:In 1995 the Tucson Children's Respiratory Study (TCRS) identified clinically distinct phenotypes amongst early wheezers; the Avon Longitudinal Study of Parents And Children (ALSPAC) has recently re-examined these. OBJECTIVES:To validate statistically derived ALSPAC phenotypes in the Southampton Women's Surv...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22766
更新日期:2013-07-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950030207
更新日期:1987-03-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24585
更新日期:2020-02-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24144
更新日期:2018-10-01 00:00:00